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Gene Review

ATP6  -  F1F0 ATP synthase subunit a

Saccharomyces cerevisiae S288c

Synonyms: ATP synthase subunit a, F-ATPase protein 6, OLI2, OLI4, PHO1, ...
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Disease relevance of ATP6


High impact information on ATP6

  • We find that mdm38Delta mitochondria have reduced amounts of certain mitochondrially encoded proteins and low levels of complex III and IV and accumulate unassembled Atp6 of complex V of the respiratory chain [2].
  • Accordingly, the rho(-)/rho degrees mutation can be viewed as a rescuing event, because this mutation prevents the synthesis of the two mtDNA-encoded subunits (Atp6p and Atp9p) forming the core of this channel [3].
  • Here we show that, following its synthesis on mitochondrial ribosomes, subunit 6 of the ATPase (Atp6p) can be cross-linked to Atp10p [4].
  • The suppressor mutation in the revertant has been mapped to the OLI2 locus in mitochondrial DNA and shown to be a single base change in the C-terminal coding region of the gene [5].
  • However, despite this over expression, the amount of the long cotranscript ATP8-ATP6 remained lower than in wild type mitochondria [6].

Biological context of ATP6

  • The ability of the rho(-) suppressor genome to express subunit 6 in an atp22 null mutant constitutes strong evidence that translation of subunit 6 depends on the interaction of Atp22p with the 5'-UTR of the ATP6 mRNA [7].
  • The mutant phenotype can be partially rescued by the presence in the same cell of wild-type mitochondrial DNA and a rho(-) deletion genome in which the 5'-UTR, first exon, and first intron of COX1 are fused to the fourth codon of ATP6 [7].
  • The COX1/ATP6 gene is transcribed and processed to the mature mRNA by splicing of the COX1 intron from the precursor [7].
  • The structural mitochondrial gene ATP6 was isolated form a mitochondrial DNA library using the oligonucleotide probe procedure [8].
  • A mitochondrial gene (denoted aap1) in Saccharomyces cerevisiae has been characterized by nucleotide sequence analysis of a region of mtDNA between the oxi3 and oli2 genes [9].

Anatomical context of ATP6

  • mRNA localization to the mitochondrial surface allows the efficient translocation inside the organelle of a nuclear recoded ATP6 protein [10].
  • For this purpose, we associated a nuclear recoded ATP6 gene with the mitochondrial targeting sequence and the 3'UTR of the nuclear SOD2 gene, which mRNA exclusively localizes to the mitochondrial surface in HeLa cells [10].

Associations of ATP6 with chemical compounds

  • 2. Consequently it was shown that the OLI2 gene product, a 20-kDa peptide with high cycloheximide-resistant label, which was generally taken to be 'subunit 6' of the ATPase, is not in fact identical to this peptide [11].
  • Biogenesis of mitochondria. Defective assembly of the proteolipid into the mitochondrial adenosine triphosphatase complex in an oli2 mit- mutant of Saccharomyces cerevisiae [12].
  • In the latter case, rho0 mutants can be isolated, upon treatment with ethidium bromide, that lack six major F0 subunits, namely the nucleus-encoded subunits b, OSCP and d, and the mitochondrially encoded Atp6, 8 and 9p [13].
  • A cDNA clone encoding human pancreatic triglyceride lipase was cloned into a yeast expression vector so that the yeast PHO1 signal peptide replaced the native signal peptide [14].

Physical interactions of ATP6

  • The lower concentration of ATPase complex in the leaderless mutants correlated with less Atp6p complexed with the Atp9p ring of the F0 sector and with accumulation of an Atp6p-Atp8p complex that aggregated into polymers destined for eventual proteolytic elimination [15].

Other interactions of ATP6

  • Measurements of the steady state levels of mitochondrial mRNA showed that the defect in subunits 6 and 8 was correlated with a modification of the expression of a cotranscript ATP8-ATP6 [6].
  • The molecular weight of these fragments depends on the map position of the mutant, increasing in the direction OLI2 leads to OLI1 [16].
  • The mutants mapped between OLI1 and OLI2 on mitochondrial DNA in a region called COB [16].
  • Based on the recombinants issued from crosses of the mutants with a triple drug-resistant strain and an analysis of the resistance markers present in sigma- clones that are effective in restoring a wild-type phenotype, the PHO1 locus has been placed in the segment of DNA located between PAR1 and OLI2 [17].
  • Two regions of the wild-type mtDNA (between cyb and oli2 and between SrRNA and oxi2) appear to be dispensable for mitochondrial function [18].

Analytical, diagnostic and therapeutic context of ATP6

  • These sequences were used to design PCR primers for the amplification of partial atp6 and cox3 sequences from other members of the Boletales and outgroup taxa [1].


  1. Use of atp6 in fungal phylogenetics: an example from the boletales. Kretzer, A.M., Bruns, T.D. Mol. Phylogenet. Evol. (1999) [Pubmed]
  2. Mdm38 interacts with ribosomes and is a component of the mitochondrial protein export machinery. Frazier, A.E., Taylor, R.D., Mick, D.U., Warscheid, B., Stoepel, N., Meyer, H.E., Ryan, M.T., Guiard, B., Rehling, P. J. Cell Biol. (2006) [Pubmed]
  3. A "petite obligate" mutant of Saccharomyces cerevisiae: functional mtDNA is lethal in cells lacking the delta subunit of mitochondrial F1-ATPase. Duvezin-Caubet, S., Rak, M., Lefebvre-Legendre, L., Tetaud, E., Bonnefoy, N., di Rago, J.P. J. Biol. Chem. (2006) [Pubmed]
  4. Atp10p assists assembly of Atp6p into the F0 unit of the yeast mitochondrial ATPase. Tzagoloff, A., Barrientos, A., Neupert, W., Herrmann, J.M. J. Biol. Chem. (2004) [Pubmed]
  5. A single amino acid change in subunit 6 of the yeast mitochondrial ATPase suppresses a null mutation in ATP10. Paul, M.F., Barrientos, A., Tzagoloff, A. J. Biol. Chem. (2000) [Pubmed]
  6. Regulation by nuclear genes of the mitochondrial synthesis of subunits 6 and 8 of the ATP synthase of Saccharomyces cerevisiae. Pelissier, P.P., Camougrand, N.M., Manon, S.T., Velours, G.M., Guerin, M.G. J. Biol. Chem. (1992) [Pubmed]
  7. The Saccharomyces cerevisiae ATP22 Gene Codes for the Mitochondrial ATPase Subunit 6-Specific Translation Factor. Zeng, X., Hourset, A., Tzagoloff, A. Genetics (2007) [Pubmed]
  8. Isolation of the ATP synthase subunit 6 and sequence of the mitochondrial ATP6 gene of the yeast Candida parapsilosis. Guélin, E., Guérin, M., Velours, J. Eur. J. Biochem. (1991) [Pubmed]
  9. Biogenesis of mitochondria: the mitochondrial gene (aap1) coding for mitochondrial ATPase subunit 8 in Saccharomyces cerevisiae. Macreadie, I.G., Novitski, C.E., Maxwell, R.J., John, U., Ooi, B.G., McMullen, G.L., Lukins, H.B., Linnane, A.W., Nagley, P. Nucleic Acids Res. (1983) [Pubmed]
  10. mRNA localization to the mitochondrial surface allows the efficient translocation inside the organelle of a nuclear recoded ATP6 protein. Kaltimbacher, V., Bonnet, C., Lecoeuvre, G., Forster, V., Sahel, J.A., Corral-Debrinski, M. RNA (2006) [Pubmed]
  11. Identity problems concerning subunits of the membrane factor of the mitochondrial ATPase of Saccharomyces cerevisiae. Somlo, M., Cosson, J., Clavilier, L., Krupa, M., Laporte, I. Eur. J. Biochem. (1982) [Pubmed]
  12. Biogenesis of mitochondria. Defective assembly of the proteolipid into the mitochondrial adenosine triphosphatase complex in an oli2 mit- mutant of Saccharomyces cerevisiae. Stephenson, G., Marzuki, S., Linnane, A.W. Biochim. Biophys. Acta (1981) [Pubmed]
  13. Suppression of rho0 lethality by mitochondrial ATP synthase F1 mutations in Kluyveromyces lactis occurs in the absence of F0. Chen, X.J., Hansbro, P.M., Clark-Walker, G.D. Mol. Gen. Genet. (1998) [Pubmed]
  14. Human pancreatic triglyceride lipase expressed in yeast cells: purification and characterization. Yang, Y., Lowe, M.E. Protein Expr. Purif. (1998) [Pubmed]
  15. The leader peptide of yeast Atp6p is required for efficient interaction with the Atp9p ring of the mitochondrial ATPase. Zeng, X., Kucharczyk, R., di Rago, J.P., Tzagoloff, A. J. Biol. Chem. (2007) [Pubmed]
  16. The mitochondrial COB region in yeast codes for apocytochrome b and is mosaic. Haid, A., Schweyen, R.J., Bechmann, H., Kaudewitz, F., Solioz, M., Schatz, G. Eur. J. Biochem. (1979) [Pubmed]
  17. Localization on mitochondrial DNA of mutations leading to a loss of rutamycin-sensitive adenosine triphosphatase. Foury, F., Tzagoloff, A. Eur. J. Biochem. (1976) [Pubmed]
  18. Elevated levels of petite formation in strains of Saccharomyces cerevisiae restored to respiratory competence. II. Organization of mitochondrial genomes in strains having high and moderate frequencies of petite mutant formation. Evans, R.J., Clark-Walker, G.D. Genetics (1985) [Pubmed]
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